title: [Inflammasomes and related diseases].
abstract: Although inflammation is important for host defense, excessive inflammation sometimes causes serious consequences. IL-1β is one of major proinflammatory cytokines. Dysregulation of IL-1β promotes development of several diseases. Mature IL-1β is produced by cleavage of its proform by a protein complex named inflammasome. Inflammasome consists of NOD-LRRs containing family (NLR proteins), an adaptor protein, and a cysteine protease caspase-1. Several NLRs can be assembled into inflammasome in response to various stimulatory signals. Genetic disorder of inflammasome-IL-1 system cause autoinflammatory diseases such as cryopyrin-associated autoinflammatory disease, familial Mediterranean fever, deficiency of IL-1 receptor antagonist, and PAPA syndrome. This article reviews recent advances in the study of inflammasome and related diseases.
authors.affiliation: Center for iPS Cell Research and Application, Kyoto University, Kyoto, Japan.
authors.forename: Megumu K
authors.initials: MK
authors.lastname: Saito
mesh_terms: D001327:Autoimmune Diseases / Q000276:immunology; D004198:Disease Susceptibility; D006801:Humans; D058847:Inflammasomes / Q000276:immunology*; D007249:Inflammation / Q000276:immunology*; D007375:Interleukin-1 / Q000276:immunology
chemical_list: D058847:Inflammasomes; D007375:Interleukin-1